Objective:

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a multisystem autoimmune vasculitis which typically involves respiratory tract and kidney. Ocular involvement is seen in half of the cases and can present as scleritis, orbital inflammation, or optic neuropathy. However, internal ophthalmoplegia is exceptionally rare presentation. We present a rare and diagnostically challenging case presented with complete internal ophthalmoplegia, revealing unexpected orbital manifestation of GPA.

Background:

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Design/Methods:

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Results:

An 84-year-old man with a history of coronary artery disease status post CABG, hypertension, and hyperlipidemia initially presented with right eye discomfort, right eye tearing, and unilateral right vision blurring. Was diagnosed with isolated right sixth nerve palsy, presumed to be microvascular in origin given his vascular risk factors. However, three months later, he returned with complete internal ophthalmoplegia of the right eye, prompting further evaluation. CT imaging revealed soft tissue thickening in the posterior right orbit extending to the orbital apex, while MRI orbits demonstrated nodular enhancement surrounding the posterior intraorbital optic nerve and obliteration of the sphenoid air cells, findings suggestive of an inflammatory or granulomatous process. Laboratory studies showed positive ANA and ANCA antibodies with normal thyroid and glucose profiles. ENT was consulted for biopsy of the sphenoid sinus. The patient was diagnosed with a variant of granulomatosis with polyangiitis (GPA) and started on intravenous steroids, resulting in marked clinical improvement.

Conclusions:

This case highlights an atypical orbital presentation of GPA mimicking microvascular cranial neuropathy. Clinicians should maintain a high index of suspicion for inflammatory etiology in patients presenting with isolated cranial neuropathies. Early recognition and targeted interventions are essential for optimizing outcomes in such unique presentations.